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Risk Stratification to Prevent SCD
in Young Athletes
Dr /Khaled Hussein,MD
Professor at Critical Care Medicine department
Consultant Electrophysiologist
Cairo University
2019
Background
 Regular sports participation is encouraged by the medical
community as part of cardiovascular prevention measures,
because it improves fitness and reduces cardiovascular
morbidity and mortality worldwide.
 A large proportion of the young population participates in
competitive or recreational sports activity
 However, for a small number of individuals who harbor
cardiac conditions, exercise can sometimes be associated
with the risk of sudden death (i.e., the exercise paradox).
Sudden cardiac death (SCD) is the most frequent medical
cause of sudden death in athletes.
The definition of SCD in athletes
 Sudden cardiac death (SCD) is an unexpected death due to
cardiac causes that occurs in a short time period (generally
within 1 hour of symptom onset) in a person with known or
unknown cardiac disease.
 Some estimates of incidence include only deaths with
exertion or shortly (< 1 hour) after exertion.
Victims of SCD among Young Athletes
in Egypt
Risk Factors of SCD
Harmon KG, Drezner JA, Wilson MG, Sharma S. Br J Sports Med. 2014 Aug;48(15):1185–92
Meagan M. Wasfy, Adolph M. Hutter, and Rory B. Weiner. Methodist Debakey Cardiovasc. J 2016 Apr-Jun; 12(2): 76–80.
Causes of SCD
Hayashi et al, Circulation Research June 5, 2015
Identifiable causes of SCD in Athletes
Ackerman et al Sudden Cardiac Death in the Young
Circulation. 2016;133:1006-1026
 More comprehensive investigations in the United States and
international populations-athletes, non-athletes, and military-
support that the most common finding on autopsy in young
individuals with SCD is actually a structurally normal heart
(autopsy-negative sudden unexplained death)
Asif & Harmon .Sports Health. 2017 May/Jun;9(3):268-279.
Screening tools for CV evaluation
Wasfy et al. Methodist Debakey Cardiovasc J. 2016 Apr-Jun; 12(2): 76–80.
Twelve-lead ECG as a screening tool
what are the problems?
 Trained athletes commonly (up to 80%) show ECG changes such
as sinus bradycardia, first-degree atrioventricular (AV) block and
early repolarization, which result from physiological adaptation of
the cardiac autonomic nervous system to athletic conditioning, such
as increased vagal tone and/or withdrawal of sympathetic activity.
 Misinterpretation of benign physiological ECG changes is not
uncommon particularly when performed by physicians without
expertise in sports cardiology
Holly RG et al,1998
Twelve-lead ECG as a screening tool
what are the problems?- cont
 False-positive ECG findings can result in unnecessary, costly
secondary investigations and disqualification from sport.
 The high false-positive ECG rates and impact on specificity are
commonly cited as major limitations of the ECG as a screening test
in young athletes
 So, an ECG criteria development to improve the efficacy of ECG
screening to acceptable levels of false-positive rates and high rates
of sensitivity to identify athletes at risk of SCD is mandatory
Drezner JA etal, Br J Sports Med. January 10, 2013
Drezner JA etal, Br J Spots Med. 2016
International consensus Standard for
ECG interpretation in Athletes
Drezner JA, et al. Br J Sports Med 2017
Echocardiography as a screening test
 The use of echocardiography as a screening test is
controversial.
 Routine use of echocardiography is expensive.
 Echocardiography is not the best modality to detect patients
with arrhythmogenic abnormalities such as ion
channelopathies and Wolf-Parkinson-White syndrome that
lead to SCD
 Echocardiography is more useful in detecting HCM and
coronary artery anomalies, which are the most common
causes of SCD in the United States
Echo Res Pract. 2018 Mar; 5(1): G1–G10.
Upper limits for ventricular dimensions
in athletes
Echo Res Pract. 2018 Mar; 5(1): G1–G10.
Echo Res Pract. 2018 Mar; 5(1): G1–G10.
Echo Res Pract. 2018 Mar; 5(1): G1–G10.
Arrhythmia in Structurally normal heart
 Congenital Long QT syndrome
 Catecholaminergic polymorphic VT (CPVT)
 Brugada syndrome
 Other ion channelopathies
Pre-excitation syndrome
 Atrioventricular (AV) bypass tracts cause early, anomalous ventricular
activation before normal activation through the AV node
 Estimated prevalence 1:1,000
 Can be asymptomatic or present with paroxysmal supraventricular
tachycardia symptoms – palpitations, syncope
 Symptomatic patients at high risk of SCD (≈0.15%/yr)
 Ventricular pre-excitation seen in ≈1% of athletes with SCD
Hiss RG, Lamb LE. Circulation 1962;25:947–61
Maron BJ et al. Circulation 2009;119:1085–92
ECG diagnosis of pre-excitation
Management of asymptomatic pre-
excitation
Long QT syndrome
 Group of disorders characterised by prolonged QT interval on ECG and
predisposition to develop life-threatening arrhythmias such as torsades de
pointes during exercise or stress.
 Prevalence of 1:2,000 newborns.
 Disease-causing mutation can be identified in 75% of patients by genetic
screening.
 Emotional stress and exercise, particularly swimming, are important triggers
for ventricular arrhythmias and SCD in LQT1, but significant overlap exists
between genotypes.
Schwartz PJ et al. Circulation 2009;120;1761–7 2
Ackerman MJ et al. Europace 2011;13:1077–109
LQT syndrome: Diagnosis
 Measurement of QTc using Bazett's formula on repeated 12-lead
ECG at stable heart rates (60–100 bpm).
 Consider LQTS if unexplained syncope and QTc >460 ms or
asymptomatic and QTc ≥480 ms
 When QTc prolongation not obvious, use risk score (age,
clinical/family history, QTc duration, T-wave morphology, previous
history of torsade de pointes); LQTS risk score >3 is diagnostic
Priori SG et al. Europace 2015;17:1601–87 2.
Schwartz PJ et al. Circulation 1993;88:782–4
Genes involved in monogenic
causes of SCD
Alfred L, George JR .J Clin Invest. 2013 Jan 2; 123(1): 75–83.
Brugada syndrome
 Characterized by right bundle branch block, persistent ST
segment elevation and sudden death due to polymorphic
ventricular tachycardia(PVT) and/or VF in absence of other
cardiomyopathies
Brugada P, Brugada J. JACC 1992;20:1391–6
Type I Brugada
Brugada syndrome
 May be accompanied by mild RV abnormalities
 Inherited disease, but may be sporadic in up to 60% of cases
 Prevalence of 1:1,000 in Asia, but <1:10,000 in Europe and
America
 Ventricular arrhythmias generally occur at rest. Chronic
athletic
 conditioning or raised body temperature during exercise may
exacerbate the condition
Rudic B et al. Europace 2016;18:1411–9
Mizusawa Y, Wilde AA. Circ Arrhythm Electrophysiol 2012;5:606−16
CPVT
 It is an adrenergic-induced bidirectional or polymorphic ventricular
tachycardia
 Prevalence around 1:10,000
 Two-thirds of cases due to known genetic mutations, Silent mutations
in 20%
 If undiagnosed, mortality rates are 30–50% by age 40
 Physical activity is a common trigger of ventricular arrhythmias in
patients with CPVT
 Diagnosis – structurally normal heart, normal ECG. Effort or emotion
trigger ventricular premature beats that increase in complexity with
heart rate
Priori SG et al. Europace 2013;15:1389–406 2.
Priori SG et al. Circulation 2002;106:69–74
BDVT VVF
Management of affected athletes with
pry arrhythmia syndrome
 Genetic testing+ family screening
 Removal of the triggers
 Beta Blockers (BBs)
 Antiarrhythmic drugs ) AADs
 Intra-cardiac defibrillator (ICD)
 Lt cervical sympathectomy
ECG-1
 Incomplete RBBB
 QRS duration <120 ms
 Normal findings in athletes
 Does not require additional evaluation
ECG-2
 SB
 Early repolarization
 LVH voltage criteria
 Common training related findings
 Does not require more evaluation
ECG-3
 Deep T wave inversion
 ST depression in lateral leads
 Bi atrial enlargement
 Left axis deviation
 Needs further evaluation
 HCM
ECG-4
 Complete RBBB
 QRS duration >120 ms
 Left axis deviation
 RA enlargement
 Additional investigations are needed
ECG-5
 Complete LBBB
 QRS duration 120 ms
 Always abnormal findings
 Needs further evaluation
Take Home Message
 Sudden cardiac death in the athlete is a rare but catastrophic
event for families, teams, leagues, and communities.
 Prevention of SCD in young athletes remains the priority of
the sports medicine community.
 Early identification of individuals at risk through screening
may be an important complementary strategy to reduce the
overall burden of SCD in young athletes.
 With a better understanding of the physiological adaptations
in athletes and the electrical consequences of such
alterations, ECG criteria development to guide physicians
has significantly improved the efficacy of ECG screening to
acceptable levels of false-positive rates and high rates of
sensitivity to identify athletes at risk of SCD.
Thank you for attention

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Risk stratification to prevent SCD in young athletes

  • 1. Risk Stratification to Prevent SCD in Young Athletes Dr /Khaled Hussein,MD Professor at Critical Care Medicine department Consultant Electrophysiologist Cairo University 2019
  • 2. Background  Regular sports participation is encouraged by the medical community as part of cardiovascular prevention measures, because it improves fitness and reduces cardiovascular morbidity and mortality worldwide.  A large proportion of the young population participates in competitive or recreational sports activity
  • 3.  However, for a small number of individuals who harbor cardiac conditions, exercise can sometimes be associated with the risk of sudden death (i.e., the exercise paradox). Sudden cardiac death (SCD) is the most frequent medical cause of sudden death in athletes.
  • 4. The definition of SCD in athletes  Sudden cardiac death (SCD) is an unexpected death due to cardiac causes that occurs in a short time period (generally within 1 hour of symptom onset) in a person with known or unknown cardiac disease.  Some estimates of incidence include only deaths with exertion or shortly (< 1 hour) after exertion.
  • 5. Victims of SCD among Young Athletes in Egypt
  • 6. Risk Factors of SCD Harmon KG, Drezner JA, Wilson MG, Sharma S. Br J Sports Med. 2014 Aug;48(15):1185–92 Meagan M. Wasfy, Adolph M. Hutter, and Rory B. Weiner. Methodist Debakey Cardiovasc. J 2016 Apr-Jun; 12(2): 76–80.
  • 7. Causes of SCD Hayashi et al, Circulation Research June 5, 2015
  • 8. Identifiable causes of SCD in Athletes Ackerman et al Sudden Cardiac Death in the Young Circulation. 2016;133:1006-1026
  • 9.  More comprehensive investigations in the United States and international populations-athletes, non-athletes, and military- support that the most common finding on autopsy in young individuals with SCD is actually a structurally normal heart (autopsy-negative sudden unexplained death) Asif & Harmon .Sports Health. 2017 May/Jun;9(3):268-279.
  • 10. Screening tools for CV evaluation Wasfy et al. Methodist Debakey Cardiovasc J. 2016 Apr-Jun; 12(2): 76–80.
  • 11. Twelve-lead ECG as a screening tool what are the problems?  Trained athletes commonly (up to 80%) show ECG changes such as sinus bradycardia, first-degree atrioventricular (AV) block and early repolarization, which result from physiological adaptation of the cardiac autonomic nervous system to athletic conditioning, such as increased vagal tone and/or withdrawal of sympathetic activity.  Misinterpretation of benign physiological ECG changes is not uncommon particularly when performed by physicians without expertise in sports cardiology Holly RG et al,1998
  • 12. Twelve-lead ECG as a screening tool what are the problems?- cont  False-positive ECG findings can result in unnecessary, costly secondary investigations and disqualification from sport.  The high false-positive ECG rates and impact on specificity are commonly cited as major limitations of the ECG as a screening test in young athletes  So, an ECG criteria development to improve the efficacy of ECG screening to acceptable levels of false-positive rates and high rates of sensitivity to identify athletes at risk of SCD is mandatory
  • 13. Drezner JA etal, Br J Sports Med. January 10, 2013
  • 14. Drezner JA etal, Br J Spots Med. 2016
  • 15. International consensus Standard for ECG interpretation in Athletes Drezner JA, et al. Br J Sports Med 2017
  • 16. Echocardiography as a screening test  The use of echocardiography as a screening test is controversial.  Routine use of echocardiography is expensive.  Echocardiography is not the best modality to detect patients with arrhythmogenic abnormalities such as ion channelopathies and Wolf-Parkinson-White syndrome that lead to SCD
  • 17.  Echocardiography is more useful in detecting HCM and coronary artery anomalies, which are the most common causes of SCD in the United States
  • 18. Echo Res Pract. 2018 Mar; 5(1): G1–G10.
  • 19. Upper limits for ventricular dimensions in athletes Echo Res Pract. 2018 Mar; 5(1): G1–G10.
  • 20. Echo Res Pract. 2018 Mar; 5(1): G1–G10.
  • 21. Echo Res Pract. 2018 Mar; 5(1): G1–G10.
  • 22. Arrhythmia in Structurally normal heart  Congenital Long QT syndrome  Catecholaminergic polymorphic VT (CPVT)  Brugada syndrome  Other ion channelopathies
  • 23. Pre-excitation syndrome  Atrioventricular (AV) bypass tracts cause early, anomalous ventricular activation before normal activation through the AV node  Estimated prevalence 1:1,000  Can be asymptomatic or present with paroxysmal supraventricular tachycardia symptoms – palpitations, syncope  Symptomatic patients at high risk of SCD (≈0.15%/yr)  Ventricular pre-excitation seen in ≈1% of athletes with SCD Hiss RG, Lamb LE. Circulation 1962;25:947–61 Maron BJ et al. Circulation 2009;119:1085–92
  • 24. ECG diagnosis of pre-excitation
  • 25. Management of asymptomatic pre- excitation
  • 26.
  • 27. Long QT syndrome  Group of disorders characterised by prolonged QT interval on ECG and predisposition to develop life-threatening arrhythmias such as torsades de pointes during exercise or stress.  Prevalence of 1:2,000 newborns.  Disease-causing mutation can be identified in 75% of patients by genetic screening.  Emotional stress and exercise, particularly swimming, are important triggers for ventricular arrhythmias and SCD in LQT1, but significant overlap exists between genotypes. Schwartz PJ et al. Circulation 2009;120;1761–7 2 Ackerman MJ et al. Europace 2011;13:1077–109
  • 28. LQT syndrome: Diagnosis  Measurement of QTc using Bazett's formula on repeated 12-lead ECG at stable heart rates (60–100 bpm).  Consider LQTS if unexplained syncope and QTc >460 ms or asymptomatic and QTc ≥480 ms  When QTc prolongation not obvious, use risk score (age, clinical/family history, QTc duration, T-wave morphology, previous history of torsade de pointes); LQTS risk score >3 is diagnostic Priori SG et al. Europace 2015;17:1601–87 2. Schwartz PJ et al. Circulation 1993;88:782–4
  • 29. Genes involved in monogenic causes of SCD Alfred L, George JR .J Clin Invest. 2013 Jan 2; 123(1): 75–83.
  • 30. Brugada syndrome  Characterized by right bundle branch block, persistent ST segment elevation and sudden death due to polymorphic ventricular tachycardia(PVT) and/or VF in absence of other cardiomyopathies Brugada P, Brugada J. JACC 1992;20:1391–6 Type I Brugada
  • 31. Brugada syndrome  May be accompanied by mild RV abnormalities  Inherited disease, but may be sporadic in up to 60% of cases  Prevalence of 1:1,000 in Asia, but <1:10,000 in Europe and America  Ventricular arrhythmias generally occur at rest. Chronic athletic  conditioning or raised body temperature during exercise may exacerbate the condition Rudic B et al. Europace 2016;18:1411–9 Mizusawa Y, Wilde AA. Circ Arrhythm Electrophysiol 2012;5:606−16
  • 32. CPVT  It is an adrenergic-induced bidirectional or polymorphic ventricular tachycardia  Prevalence around 1:10,000  Two-thirds of cases due to known genetic mutations, Silent mutations in 20%  If undiagnosed, mortality rates are 30–50% by age 40  Physical activity is a common trigger of ventricular arrhythmias in patients with CPVT  Diagnosis – structurally normal heart, normal ECG. Effort or emotion trigger ventricular premature beats that increase in complexity with heart rate Priori SG et al. Europace 2013;15:1389–406 2. Priori SG et al. Circulation 2002;106:69–74
  • 34.
  • 35. Management of affected athletes with pry arrhythmia syndrome  Genetic testing+ family screening  Removal of the triggers  Beta Blockers (BBs)  Antiarrhythmic drugs ) AADs  Intra-cardiac defibrillator (ICD)  Lt cervical sympathectomy
  • 36. ECG-1  Incomplete RBBB  QRS duration <120 ms  Normal findings in athletes  Does not require additional evaluation
  • 37. ECG-2  SB  Early repolarization  LVH voltage criteria  Common training related findings  Does not require more evaluation
  • 38. ECG-3  Deep T wave inversion  ST depression in lateral leads  Bi atrial enlargement  Left axis deviation  Needs further evaluation  HCM
  • 39. ECG-4  Complete RBBB  QRS duration >120 ms  Left axis deviation  RA enlargement  Additional investigations are needed
  • 40. ECG-5  Complete LBBB  QRS duration 120 ms  Always abnormal findings  Needs further evaluation
  • 41. Take Home Message  Sudden cardiac death in the athlete is a rare but catastrophic event for families, teams, leagues, and communities.  Prevention of SCD in young athletes remains the priority of the sports medicine community.  Early identification of individuals at risk through screening may be an important complementary strategy to reduce the overall burden of SCD in young athletes.
  • 42.  With a better understanding of the physiological adaptations in athletes and the electrical consequences of such alterations, ECG criteria development to guide physicians has significantly improved the efficacy of ECG screening to acceptable levels of false-positive rates and high rates of sensitivity to identify athletes at risk of SCD.
  • 43. Thank you for attention